The Nutritional Management of People with Huntington’s Disease (HD): A Practical Handbook for Carers and Dietitians
Reference number:
NMH-HD-DIET-SoC-280310
Issue:
Issue 1 Draft D final
Prepared by:
Lillian Campos, Arleen Rowell, Ailsa Brotherton.
Date:
September 2011
Acknowledgments The EHDN-Hd SOC Dietitians Group have provided expertise in the development of the handbook which aims to share practical advice and strategies surrounding nutritional issues in HD. In particular we are very grateful to patients, their families and the staff at Frank Gardham House, A Specialist Unit for the Care of People with HD, Lancashire Care Foundation Trust, Preston, Lancashire U.K. This handbook has been produced to support the European Huntington’s Disease Network (EURO-Hd), Standards of Care (SOC) Nutritional Guidelines for People with HD. Therefore, it should be read by clinicians in conjunction with these guidelines (1). © 2011
Abbreviations
AD ETF EURO-Hd HD PEG PPIs GORD OT SSRIs SOC SLT NGT
Advance Directives Enteral Tube Feeding
European Huntington’s Disease Network Huntington’s Disease Percutaneous Endoscopic Gastrostomy Protein Pump inhibitors
Gastroesophageal reflux disease Occupational Therapist Selective serotonin re-uptake inhibitors Standards of Care Speech and Language Therapist Nasogastric Tube
Contents INTRODUCTION ____________________________________________________________________________2 PART 1: NUTRITION AND HUNTINGTON’S DISEASE _____________________________________________4 1.1 1.2 1.3 1.4
HD AND W EIGHT LOSS __________________________________________________________________4 SYMPTOMS ___________________________________________________________________________5 IMPACT OF SYMPTOMS OF HD ON EATING AND DRINKING _________________________________________5 COMMUNICATION WITH HD PATIENTS________________________________________________________6
PART 2: NOTES FOR CARERS _______________________________________________________________7 2.1 COMMON PROBLEMS AT MEALTIMES ________________________________________________________7 2.1.1 PHYSICAL FACTORS ____________________________________________________________________7 2.1.2 Cognitive Factors _______________________________________________________________ 11 2.1.3 Emotional Factors Table _________________________________________________________ 12 2.2 THE VALUE OF FOOD __________________________________________________________________ 13 *NOTE_________________________________________________________________________________ 13 2.3 BALANCED DIET _____________________________________________________________________ 14 2.3.1 Balanced Diet _________________________________________________________________ 14 2.3.2 How to Enrich & Adapt ___________________________________________________________ 16 2.3.3. Blended high calorie milk shakes** ideas __________________________________________ 17 2.4 PUREED DIETS ______________________________________________________________________ 18 2.4.1 Further ideas for enriching a pureed diet (to advised consistency): ________________________ 19 Iron intake ___________________________________________________________________________ 20 2.4.3 Regular Aspects __________________________________________________________________ 20 DIABETES _______________________________________________________________________________ 22 2.6 ADVANCED DECISIONS (AD) AND ENTERAL TUBE FEEDING (ETF) _________________________________ 2.6.1 Enteral Tube Feeding (ETF) ________________________________________________________ Types of Tube ________________________________________________________________________ 2.6.2 Mode of feed __________________________________________________________________ 2.6.3 Care of Percutaneous Endoscopic Gastrostomy (PEG) ___________________________________ Tube Care Do’s and Don’ts: _____________________________________________________________ 2.6.4 Percutaneous Endoscopic Gastrostomy (PEG) _________________________________________ Balloon Gastrostomy___________________________________________________________________ 2.6.5 Flushing the Enteral Feeding Tube (EFT) ______________________________________________ 2.6.7 Use of clamps ___________________________________________________________________ 2.7. ENTERAL TUBE FEEDING (ETF) GASTROINTESTINAL PROBLEMS IN HD _____________________________ 2.7.1. Vomiting/Gastrointestinal reflux (GORD) ______________________________________________ 2.7.2 Diarrhoea _______________________________________________________________________ 2.7.3 Constipation _____________________________________________________________________ PART 3:
23 23 23 24 24 24 25 25 25 26 26 26 27 27
NOTES FOR PRACTICING DIETITIANS ____________________________________________ 28
3.1 HD DIETETIC ASSESSMENT FRAMEWORK___________________________________________________ 3.2 NUTRITIONAL CARE PLAN ________________________________________________________________ 3.3 MULTIDISCIPLINARY WORKING ___________________________________________________________ 3.4. MAKING AN INFORMED CHOICE ON ENTERAL TUBE FEEDING (ETF)______________________________ 3.5 ENTERAL TUBE FEEDING (ETF) DECISION MAKING CHECK LIST __________________________________
Appendix 1 _______________________________________________________________________________________ Appendix 2 _______________________________________________________________________________________ Appendix 3 _______________________________________________________________________________________ Appendix 4 _______________________________________________________________________________________ Appendix 5 _______________________________________________________________________________________
28 29 30 30 32
33 34 40 41 42
1
Introduction Weight loss is very common in people with HD despite the fact that they commonly have very good appetites. One of the biggest challenges to those who care for people with HD can be preventing weight loss and helping them to maintain a healthy weight. The intention of this handbook is to support Carers and Dietitians who are involved in the nutritional management of those with the HD gene. The main focus of the handbook is on preventing weight loss. It is hoped that it will be especially useful for those who have not had the opportunity to build up experience and expertise in this area. The handbook is organised into 3 parts each considering different aspects of nutritional management of people with HD. The first part is aimed at all who are involved in the care or management of those with HD. The second part is aimed at carers and the third is aimed at Dietitians. However it is hoped that the handbook will be a useful resource to all readers. An overview of each Part is provided below. Part 1: A description of the relationship between nutrition and Huntington’s disease
A discussion of HD and its symptoms, how these symptoms can impact on weight changes and the importance of preventing weight loss.
An overview of how the symptoms of HD may lead to challenges for the individual at mealtimes and the importance of carers recognising these challenges ,
The need for good communications skills in order to offer the best support and some suggestions for effective communication
Part 2: The role of the Carer
Highlights the value of allowing people with HD to eat independently in order to enjoy mealtimes, and maintain their self esteem, and includes practical strategies for carers to support them to achieve this. It also shares problem solving ideas surrounding common nutritional issues.
Discusses the importance of social interaction as well as the nutritional value of food.
Gives guidance on how to achieve a balanced diet, enriching and adapting meals where necessary in accordance with any texture modification required, as well as guidance on iron intake. The importance of routine in the day to day life of people with HD is also emphasised.
Some guidance is given on HD and other clinical conditions affected by diet
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Discusses the value of advance decision on Enteral Tube Feeding (ETF) to ensure the patients wishes are known while they have the capacity to make decisions regarding their care .
Highlights specific issues related to Enteral Tube Feeding and gives some trouble shooting advice.
Part 3: Notes for practising Dietitians
Highlights the variability of energy requirements, advises early dietetic intervention and regular reviews of care plans. The importance of building trust with patients and carers is emphasised
Describes the main elements of a comprehensive nutritional assessment framework and care plans and highlights the benefits of multidisciplinary working.
Highlights the importance of enabling the patient to make an informed choice on Enteral Tube Feeding ( ETF) based on their own values and discusses the importance of procedure in the decision making process.
Guidance is given on how the Dietitian can help people with HD make an informed choice on long term feeding plans while they have the capacity to do so. Some important points to convey are highlighted.
Suggests areas to be explored in the decision making process for ETF when the patient lacks capacity
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Part 1: Nutrition and Huntington’s disease
1.1
HD and Weight Loss
Huntington’s disease [HD] is a familial neurodegenerative disease with an autosomal dominant inheritance (2, 3).This means that each child of an affected parent has a 50% chance of inheriting the disease. HD usually develops between the ages of 30 and 50, but it can appear in older adults and in children. The symptoms are uncontrolled movements, abnormal balance when walking, slurred speech, difficulty swallowing, thinking difficulties, and personality changes. The symptoms gradually get worse, sometimes over a period of up to 20 years leading to complete dependency. The order in which the symptoms of HD develop and the rate of the progression of the disease varies from person to person. Currently there is no cure for HD but there is a great deal of research being carried out .We now also understand more about factors which can help to improve the quality of life for people with this condition. Both good nutrition and enjoyment of mealtimes play a key role in everyone’s quality of life. This is particularly true for those with the HD gene who generally have very good appetites. However, eating, drinking and maintaining a healthy weight can be one of the biggest challenges for them and those who care for them. Weight loss is common in people with HD despite a high calorie intake. Several factors may contribute to weight loss. It may be due to increased calorie requirements and insufficient intake, altered metabolic function (4) or swallowing problems (5). When weight loss occurs it can be very rapid so preventing weight loss and maintaining a healthy weight is very important. However the changes that occur in o the HD individual as the disease progresses and symptoms appear have a profound effect on food intake. To understand these changes it is generally agreed that the symptoms may best be understood as three overlapping areas: physical, cognitive and emotional. (6)
Physical
Cognitive
Emotional
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1.2
Symptoms
Physical The main physical problems are difficulties with motor coordination and muscle control which may result in uncontrolled movements and difficulty walking, speaking and swallowing. Uncoordinated movements are a challenge when handling cutlery and taking food to the mouth. Chewing and swallowing difficulties arise from uncoordinated movement of the face, neck, lips and diaphragm. This can make eating and drinking a tiring experience. Other common problems are vomiting, reflux and regurgitation of food as the systems for moving food through the digestive system become disrupted. Cognitive Cognitive changes, which are to do with the ability to think, impact on problem solving, planning and sequencing of ideas. This includes the decisions before the food enters the mouth and may lead to an inappropriate food choice and/or eating too much too quickly. Concentration and memory can also be affected. Overall, an individual’s awareness and ability to make judgments become more impaired. Emotional Mood swings and changes in emotional responses are common, all of which can adversely affect behaviour. There is no psychiatric disorder specifically associated with HD, although depression is often reported. This may not only be because of the burden of having a progressive disorder but also as a direct result of damage to certain brain cells. Depression can affect the person’s appetite and anxiety can make movements more pronounced and affect the swallowing process. Frustration may occur when faced with being unable to prepare food and eat in the usual way. Many people with HD have a great resistance to adaptive changes to their lifestyle which can make accepting advice for safer eating or altering the texture of food and fluid difficult.
1.3
Impact of symptoms of HD on Eating and Drinking
It is important for Carers and Health Professionals to recognise the many challenges that the HD individual may encounter at meal times in order to help them to overcome, or work round some of these problem areas. However, identifying the best form of support is not always straightforward as difficulties often arise as a result of the interaction of several factors. For example a physical difficulty may cause problems with using a knife and fork. This might lead to frustration and trigger an emotional response or the person might feel that mealtimes are now too much of a challenge and so avoid them. Cognitive changes make routines very important to the HD individual and impatience with any delays is often exhibited. In addition, too much visual and auditory stimulation can cause anxiety and confusion and lead to a temper outburst and a meal refused. Anxiety may also lead to increased movement and result in food spillage as well as causing increased difficulty swallowing or increase the risk of coughing at mealtimes. Therefore providing the best support to alleviate physical problems and keeping mealtimes structured in a calm environment are very important. The emotional behaviour caused by the disease process is not always under the control of those with HD. We must be aware of the possibility that underlying factors , unable to be articulated , may be the cause of apparently unreasonable behaviour. Understanding the 5
causes of behaviour helps us to take a more holistic approach and avoid causing unnecessary additional stress to people with HD by misinterpreting the person’s behaviour. In addition to understanding behaviour, practical strategies to support behaviour are crucial. Some examples of common problems encountered at mealtimes with strategies to help overcome these are suggested in Section 2.1. However, firstly we must look at ways to improve communication, as good communication skills are fundamental to identifying an individual’s needs.
1.4
Communication with HD Patients
People with HD may have difficulties speaking clearly and in starting a conversation. You can improve communications by applying simple techniques, such as:
Make them feel comfortable and relaxed. Greet them by their name, using a calm tone and by positioning yourself at eye level Position your body correctly Think about your body position e.g. it may be better if you are positioned at a 45 degree angle and not directly in front of them. Use of body language Open hand gestures referred to as showing palm communicates both honesty and a non threatening approach 7). Patience If you ask a person with HD a question give them plenty of time to reply. If you repeat the question while they are forming a reply you will delay the response even more. Repeat back Ungrammatical sentences are common e.g. the word order may be incorrect or a word or verb might be omitted. Keep it simple Don’t ask more than one question at a time and keep to one topic of conversation at a time. Prompt the conversation Encourage two way conversation as much as possible. Provide assistance where required. Keep your request brief and give cues to help them along e.g. do you want mince or fish, would you like coffee or orange juice? This cueing can assist the patients with word finding. Or ask them to give you the first letter of a word (8) Be honest. If you don’t understand what is being said ask them to repeat it or to spell the word for you. If you cannot understand what is being said try to describe what they are trying to say or indicate the first letter of the word. You can try using gestures, word boards; picture boards (9). Remove distractions For example, turn off the television, if this doesn't provoke agitation. Never give up. Even when communication is difficult, set up a signalling system that will allow the person with HD to communicate nonverbally. Make flashcards of specific objects or use objects which they can point to(10). Keep your humour Using humour, and sharing laughter with someone is valuable in terms of building trust, and can provide a distraction in times of stress. Often the use of humour is the only form of therapy that someone may need at a given time. Don’t stop conversation Even in the late stages when a person cannot express themselves it is extremely likely that they can still understand what is being said (9). Including them in conversation at mealtimes (or at any time) or describing what they are about to eat ,for example ,may help to improve the mealtime experience. It may also give reassurance or reduce feelings of isolation that may result from the loss of speech
See EURO-HD SLT SOC guidelines (11)
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Part 2: Notes for Carers 2.1
Common problems at mealtimes
It is important that mealtimes continue to be a source of enjoyment despite the problems encountered. Carers should try to support people with HD to eat and drink independently for as long as possible as this will help them to maintain quality of life and self-esteem. The information below will assist carers to do this. They outline common barriers and behaviours witnessed at mealtimes and provide practical solutions to support the HD individual. All food and fluids given to the HD individual must be appropriate for their chewing and swallowing ability and follow any advice given by the Speech and Language Therapist (SLT) For convenience, the information has been grouped into three areas relating to physical, cognitive and emotional factors. However, it is important to remember that observed difficulties may not always arise from one single factor but may be a combination of contributing causes. 2.1.1 Physical Factors Food spillage due to poor coordination
Monitor difficulties with utensils used for eating and drinking. Use large-grip handled cutlery, non-slip plates with raised edges, soup plates or ‘pasta’ plates, use cups with a cover and/or a straw. Caution: Spouted cups, straws, ‘sports’ cups tend to give less control of flow and may provoke coughing and choking. Use non-slip mats under plates, plastic table cloths can be useful and warm plates will not slip on these . Many people with large arm movements manage well using normal cups only half-filled. Consider softer texture e.g. plastic cup if there is a risk of bruising to the lips When seated ensure good support for feet and bottom with lower back against the chair. . Protect against spillage, if an apron is unacceptable to the individual offer another covering or napkins whenever needed. Aim to maintain dignity and ask permission to wipe someone’s face or leave plenty of napkins on the table and encourage them to do it themselves. Coordination of movement can worsen when tired so try to ensure regular sleeping routine or a rest before meals (don’t let meals go on for too long, about 20 minutes so food doesn’t get cold, but never hurried!). Movement can worsen with excitement or anxiety. Avoid over stimulation just before meals. If anxiety is a factor try a hand massage or passive limb exercises before meals. Relaxation before meals can also help aid digestion. Ask your OT or Physiotherapist for advice Some people find putting their elbow on the table and resting the head on one hand helpful while eating Don’t be afraid to ask if they would like some assistance (especially towards the end of a meal). If the head is unsteady while feeding try placing the palm of one hand to the person’s forehead or behind the neck or head. Fear of choking or making a mess can cause anxiety and this may increase movements and affect the swallowing process. Bear these factors in mind. 7
Taking a long time to eat Consider the texture, finely chopped moist food reduces the need for chewing. People with HD may face difficulties adjusting to different textures and may do better if they finish each item on the plate in turn. Massaging the cheek can stimulate and/or facilitate chewing. Check that cups are lightweight (though some people may benefit from a weighted cup Check for fatigue –high calorie drinks may be less tiring to manage at times when fatigue is a problem. Smaller and more frequent meals and nourishing snacks may help. Use a warming tray to keep food warm or offer a small portion, then second helpings . Give prompts/reminders to chew and swallow Mixed consistencies and food containing hard pieces (cereals with fruit, ice cream with nuts, lumpy soup) are difficult to manage and should be avoided . Whole meal bread takes less time to chew than white bread (less doughy) it is also best to cut the crusts off bread. Some assistance may be needed as the meal progresses, monitor for increased difficulties with utensils. Some food may be retained in the mouth after swallowing. Check mouth for any food residue after meals (see oral health) . Some medications for mood will dry up saliva and this can make swallowing more difficult. Interventions used are frequent sips of drink, mouth swabs( we’ve avoided these b/o risk of biting on them) artificial saliva. Maintaining an upright position while feeding Upright is the best position for eating. Never offer food or drinks while someone is lying down or in a reclining position. The person should be seated at a 90 degree angle and if possible to lean slightly forward. Feet should be flat on the floor and elbows can be placed on the table to stabilise the body. If the person has to remain in bed at meal times ensure that the head of the bed is elevated to a 45-90 degree angle using pillows or foam wedges for stability.
Leaning to side or far forward in chair Use padded support for head and trunk. Sliding down in chair Check that the seat length is not too long, lower back should be against the chair with good support for feet and bottom. A lap tray or table may provide upper body support . Use padded support where needed to keep the body on a good postition Specialised seating may need to be considered. Ask for advice from an occupational therapist or physiotherapist to help with posture or specialist seating. Difficult to keep the person’s head upright Use cushions or a blow up travel neck cushion See EURO-Hd Occupational Therapists , (12) and Physiotherapy SoC for guidance (13)
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Mouthfuls too big/eating too quickly Eating too quickly can cause problems with coughing and choking. Give instructions about the chewing movement to eat slowly and deliberately . Slow down the rate by advising to alternate sips of fluids with bites and encourage to put the cup or utensil down after each mouthful. A smaller eating utensil such as a teaspoon or two pronged fork may help.. Drinking too fast and taking large gulps can lead to swallowing air causing trapped wind, and vomiting. Try to encourage proper breathing at mealtimes to avoid swallowing too much air and aid the swallowing process. Encourage the following: breathe out before placing food in the mouth; breathe through the nose; chew food and pause; stop breathing and swallow (you cannot breath and swallow at the same time). If there is a tendency to hyperextend the neck encourage to point the chin towards the chest as this position to helps to control the rate of intake (if big gulps are taken). For some individuals using a straw may help if the fluids do not need to be thickened or use small cups for drinks, and offer more often. Make sure that food/fluids are at an appropriate temperature - not too hot (but not tepid) as sensitivity to high temperatures may be reduced. If able to take a normal consistency diet ,grainy items such as ground beef or rice may irritate the pharynx and cause coughing or choking and may need to be avoided or served with a thick sauce. For those who cough easily spicy soups and food with lots of vinegar can tickle the throat and cause coughing, though do not over restrict if it’s not a problem for the individual . Position the chair so that there is easy access in case of a choking episode. If choking occurs never use fluids to wash down food, use back slaps and abdominal thrusts to dislodge any food items if necessary (14) Carers should be aware of how to manage a choking incident and undertake these methods effectively. Chewing and swallowing problems Self feeding Speech and Language Therapists’ advice on the texture of meals and drinks should always be followed carefully. As difficulties become more severe, foods may increasingly need to be modified. Initially moistening and cutting up may be all that is needed. Generally a moist-mash (i.e. smooth and moist texture without lumps) is easiest to chew. Encourage independence for as long as possible as self feeding is usually safer than being fed by someone else. Over time more assistance may be needed, for example to load the spoon, but continue to promote involvement in taking the spoon to the mouth. Sharply flavoured foods and drinks like cranberry or lemon juice, and drinks that are hot (but not tepid) or chilled can stimulate a stronger swallow because they provide more stimulation than bland and tepid food and drinks. Encourage people with HD to close their mouth firmly around spoon before they take it away from their mouth. Give verbal cues e.g. instruct to chew and swallow deliberately before the next mouthful. Brief comments help to make mealtimes a sociable occasion, but avoid asking questions which require an answer as speaking while eating will increase the risk of choking. Medications can dry the mouth and make swallowing difficult (review the need for medications). 9
Minimised distractions to help individuals concentrate on chewing and swallowing. Fed by others
Feeding someone with HD can take a long time and sufficient time needs to be set aside for this. The quality of the relationship between the person feeding and person being fed will have an impact on the amount of food eaten. The person feeding should be clearly visible to the person with HD. Show the spoon clearly while bringing it towards the mouth, put spoon deliberately on the tongue pressing gently and ask the patient to close lips around the spoon before removing it. Never scrape the spoon off the top teeth. Anxiety can cause problems with swallowing and result in coughing, try to use calming techniques such as hand massage, deep breathing before meals and give reassurance during meals. As swallow becomes more compromised due to the progression of HD, fatigue, infection, or other psychological factors, the risk of aspiration, chest infections and pneumonia increase.
Both self-feeding and fed by others
Preparation and presentation of food is very important. It is essential to ensure that if a soft or pureed food has been advised this is what is provided. To enhance the appearance and enjoyment of different flavours at a meal, ensure that foods are served separately, unless the person requests them mixed together. Mixing flavours could cause confusion to someone who is cognitively impaired. Refer to SLT if there are any signs of swallowing worsening e.g. taking a longer time to swallow, coughing at meal times, food remaining in the mouth for a long time, change in breathing pattern, a high temperature which may indicate a chest infection. In all cases respect a refusal, try to achieve calm and restart the meal with alternative choices.
Weight loss Careful supervision is needed of both the swallowing process and any changes in food intake, or weight changes More fatigued at meal times? They may have a poor sleeping pattern and be waking during the night (If so, encourage rest between meals and make sure meals are small but enriched with extra calories. See Enriching meals (Section 2.3.2. ). If waking during the night offer a nutritious warm drink such as soup or a soup type nutritional supplement. Milky drinks can be offered if these are preferred but avoid adding extra sugar to drinks (the natural defences against tooth decay are slowed down during the night). Are they sleeping late and missing breakfast? If so make up for this with high calorie snacks between meals and at bedtime. Is there a problem with constipation? Constipation can reduce appetite; chart the fluid intake as they may be drinking less. Encourage fibre containing foods and regular fluids Increased problems with utensils/self feeding? Offer help at mealtimes if there are signs that it is needed - help may only be needed towards the end of the meal. Is the swallow worsening? Fear of choking can result in avoidance of certain foods. Monitor for difficulties with swallowing such as taking longer over meals, e.g. coughing at meal times, high temperature, change in breathing pattern, chest infection. Refer to SLT if there are any 10
signs of swallowing difficulties. Is it due to eating less than previously? Chart food and fluid intake taking into account food spillage. If eating less refer to Enriching meals Section 2 Weight loss continuing despite enriching meals? Offer nourishing snacks/milk shakes between meals. Nutritional supplements may be required. If weight loss continues contact your Dietitian or refer to the supplement flow chart, appendix 1 for guidance. Vomiting/GORD Vomiting, reflux of food into the oesophagus from the stomach and regurgitation of phlegm are common problems in HD. Anti-reflux medications and medications to help the stomach to empty sooner are useful. (see gastrointestinal problems of tube feeding). (see section 2.5.1)? Ensure meals are eaten in an upright position and avoid over filling the stomach at mealtimes e.g. smaller more frequent meals will help. Sips of fluids only at mealtimes - and give full drinks at least a half hour after meals. Avoid lying down immediately after meals (for at least a half hour). Monitor for constipation. Avoid anything that restricts the stomach i.e. tight fitting clothes or belts. Monitor for ‘Eating too quickly’: and ‘Drinking too fast’. Relaxation before and after meals may help. Eating requires a lot of concentration so turn TV and radio off – if this is acceptable. If excessive weight gain is a problem this can increase the risk of GORD reduce the total amount of fat and sugar offered. (see Appendix 1 for some guidance) See section on Enteral tube feeding if relevant.
2.1.2 Cognitive Factors Reduced ability to plan and problem solve may lead to difficulties shopping and preparing meals If helping someone to prepare meals: Help to make lists of tasks to do; Keep menus simple; Stickers on kitchen doors helps to locate items; Give no more than one verbal instruction per action; Prompt each step of an activity ; May not recognise own disabilities so observe closely around hot cooking equipment, sharp knives etc for unsafe behaviour. Eating requires a lot of concentration and energy Help to prevent reduced attention and concentration which may result in meals left unfinished : Avoid interrupting the patient or asking questions while they are eating; Environment should be free of distractions; Observe and offer help when needed; Give gentle reminders to eat if necessary. Behavioural issues Fixing on a few preferred foods may have a potential for deficiency if a food group is missing. Try to encourage variety as much as possible (see 2.2) or ask for a Dietitian’s advice to check that the overall diet is balanced and adequate. There may be an altered sleep pattern so that night time is when extra snacks can be 11
offered to compensate for daytime meals/drinks being missed. 2.1.3 Emotional Factors Table Changes in mood Anxiety & Anger Prepare for mealtimes and have appropriate feeding aids on hand and communicate what food is being offered e.g. ‘today we are having chicken’. Routines are very important so establish routines and stick to them. In general it’s best to avoid surprises. The environment should be calm, soft background music may help some, and structured with no interruptions during mealtimes. Mealtimes should be unhurried. If the meal is going to be delayed communicate this to reduce anxiety or frustration. Acknowledge any anger and try to shift their focus to something they enjoy. Listen and show patience to find out the cause, prevention is always the best way. Consider potentially reversible causes of restlessness and agitation such as infections, pain, constipation, electrolyte imbalances (?), hypoglycaemia, hypoxia, drugs (anticholinergic, opiods, benzodiazepines, and antipsychotics), drug withdrawal (including cigarettes and alcohol). (15) Fretting Avoid discussing future events too soon as this may cause fretting. Try to reassure and shift focus to a favourite food or activity. Apathy or lethargy There is an inability to initiate so prompt at mealtimes to help them to get started. Gently coax/guide behaviour but respect “no.” Excitability Watch for triggers (If there is something that triggers it then take steps to avoid this in the future). Check caffeine intake and consider caffeine free drinks if intake is excessive. Food refusal Consider the food being offered. Does the meal look appetising? Are all foods being refused or is there a particular one? Is this a favourite food? Do they tend to eat better when no-one is watching? Would they eat more in private or when observed at a distance only? Supervision will still be needed when someone has dysphagia. Check the environment – is it calm? Is there any indication of constipation? (check fluid intake). Is it a particular texture that is being refused? Are the foods mixed together? Staring at food without eating may be an indication of increasing difficulties swallowing and fear of choking. Is there a need to request SLT review of swalowing ability? Is there a need to have mood assessed by a health professional? Consider the whole picture - is the problem physical, cognitive, emotional?
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2.2
The value of food
We often talk about the nutritional value of food and we know that what we eat and drink has an essential role in maintaining good health. However food brings more than just nutritional benefits to the table. The social interactions experienced through sharing mealtimes with friends, companions and carers alike provides nourishment to body and mind alike. This is particularly relevant to those with the HD gene. and mealtimes should be viewed by carers as a therapy that contributes to the quality of life for those with HD and should never be undermined. Every effort should be made to promote mealtimes as an enjoyable and relaxed experience without pressurising individuals on the need for calories and specific nutrients. Try to follow the principles of a balanced diet, offering flavourful familiar and favourite foods. To help meet the nutritional needs of those with HD
BEAR in mind the following
Balanced Try to follow the general principles for a balanced diet using everyday, familiar foods.
Enrich If weight loss is a problem then extra calories need to be added to the daily diet. This can be done by enriching the diet, for example with extra grated cheese, cream, spreading fats or oils and/or including extra snacks or high calorie milk shakes between meals.
Adapt Taste preferences seldom change in people with HD as the disease progresses so adapt favourite foods and snacks. Try to incorporate meals that can be enjoyed by the whole family and adapted to suit the needs of the person with HD
Regular Regular meal pattern is very important. Regular bowel movements to prevent constipation. Regular weight checks to monitor for weight loss. Regular tooth brushing for healthy teeth and gums. Regular review of medications. *Note
Excessive salivation or drooling (sialorrhea) can sometimes be a problem. There are some medications to treat this such as Glycopyrrolate or a scopolamine (hyoscine) patch but medications may bring unwanted side effects. Check for causes such as gum disease, enlarged tonsils, nasal bock due to sinusitis. Acid reflux issues and some medications, antiseizure or anti-psychosis medicines (especially clozapine) can also lead to increased secretion.
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2.3
Balanced diet
2.3.1
Balanced Diet
General principles of a balanced diet are illustrated by the Eat Well Plate:
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The table below provides examples and guidance on daily amounts for each segment of the Eat Well Plate. Food groups Bread, rice, potatoes, pasta and other starchy foods provide energy Fruit and vegetables provide fibre and vitamins and are low in calories Milk and dairy foods provide calcium and protein. Full fat options are higher in calories Meat, fish, eggs, beans and other sources of protein to maintain and repair tissues Food high in fats and sugar Provide concentrated calories and help maintain weight and energy levels
examples Potatoes, breads, cereals, rice, pasta, chapatti, polenta
How much? Include some at every meal
Fruits, vegetables (fresh, frozen, cooked or raw), fruit smoothie, Fruit juice or vegetable juices Milk, Yogurt, cheese, fromage fraise,
5 or more portions, these can be bulky so caution with portion size if appetite is poor 2- 3 times a day or more
Meat , fish, eggs, lentils, beans, peas, nuts, Soya, quorn, tofu
Include in main meals
cream, margarine, mayonnaise, *Cake, *desserts,* biscuits. *sugar, ,* jam, * syrup,
According to need. Use to enrich other foods when weight loss is a problem. (When weight loss is not a problem avoid foods high in *sugar as snacks to reduce the risk of dental caries). If weight gain is a problem these foods should be limited. See Appendix 1 8 – 10 glasses More in hot weather or in times of illness (with raised temperature) or if there are increased losses e.g. due to diarrhoea, poor lip seal that results in drooling.
Fluids Tea, milk, fruit juice and Essential to remain hydrated, a squash, water, decaffeinated poor intake will affect mental and coffee physical ability
Note 1: High risk foods such as: Stringy, fibrous texture foods can be problematic for people with swallowing difficulties and should be avoided. See Appendix 2 Table 1
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2.3.2 How to Enrich & Adapt As the condition progresses extra nourishment may be required to maintain weight - then Enrich and Adapt meals. Reduced calorie or low fat foods promoted for the general public are not appropriate when there is unintentional weight loss and extra calories are needed. However it is important to refer to Regular Dental Care 2.4.3 when offering foods that may be high in sugar** Example of a daily menu Breakfast Cereal with milk, toast with 8.00 jam and butter Orange juice Coffee Mid Morning 10.30.
Cake and cup of tea
Lunch 1.00
Soup Whole meal sandwich (egg ) Fruit crumble and custard Fruit juice
Mid Afternoon 15.30 Dinner 18.00
Supper 19.00
Fruit yogurt and cup of tea
Lamb stew & potatoes, peas and carrots Ice cream Fruit juice Horlicks (milky drink)
Ideas for increasing calorie intake Use fortified milk (see Table 5) in cereals Use spreads generously Make coffee with whole milk - try cappuccino or latte Add cream or butter to cake Use fortified milk in tea or offer milk shake** instead of tea Add cream, crème fraiche or grated cheese to soup Add mayonnaise or mashed avocado to fillings . spread margarine thickly Make custard with full fat or cream. Add ice cream**/double cream to desserts Adapt juice to a fruit smoothie** by adding to a yogurt and mixing) Use thick and creamy yoghurt or mousse** Replace yogurt with milk shake** (see recipe) Add butter & cream to potatoes & butter to vegetables Mix soft fruit or chocolate sauces** or whipped cream into ice cream** A crustless wholemeal sandwich filled with cream cheese, hummus, creamed avocado, pate or other soft moist filling.
Estimated nutritional value using average Estimated nutritional value using average portion sizes portion sizes Approximately: Approximately Energy kcal =2320 Energy kcal = 4000 Further Ideas to boost the calorie intake to the Table 5 menu:
Fortify milk by adding 2 tablespoons of dried milk powder to normal milk, shake well and use in tea, coffee, desserts Using single cream in cereals Be generous with spreads and sandwich fillings e.g. mayonnaise, salad cream, guacamole, hummus, smooth peanut butter, taramosalata, Cakes, crumpets, pancakes with plenty of butter,cream seedless jam**, lemon curd** Choose thick and creamy yogurts**, mousses**, fromage frais** as snacks( if cleaning teeth is problematic then choose low sugar but high in fat products to reduce the risk of dental caries ) 16
Add cream to coffee
2.3.3. Blended high calorie milk shakes** ideas
Chocolate milk shake Chocolate powder 50g Ice cream 500g 250mls milk 40 g whipping cream Approximately = 800mls 3 servings = 465 kcals per serving
Apple Pie Deluxe 250g Apples 150g cream 200g ice cream 2g cinnamon Approximately 600mls 2 servings = 395 kcals per serving
Café Maria 10g instant coffee 20g chocolate powder 100mls milk 100mls condensed milk 100mls whipping cream Approximately =300mls 1 serving = 795 kcals per serving
Chocolate gateau smoothie 1 large chocolate Swiss roll 15g chocolate powder 400mls whipping cream 200mls milk Approximately= 900mls 3 servings = 855 kcals per serving
Peanut butter milk shake 240mls milk 500g ice cream 130g smooth peanut butter 25 g honey or syrup Approximately 850 mls 3 servings = 625 kcals per serving
Banana Split (any soft fruit) 2 small bananas 200mls condensed milk 200mls whipping cream 50g vanilla ice cream Approximately = 600mls 2 servings = 750kcals per serving
*Your SLT may advise fluids to be thickened for safer swallowing. Ensure that all fluids are given to the advised consistency. Liaise with your SLT and see Appendix 2 Table 2 for further guidance Milk shakes are a lot of fun for the person with HD to make and even more fun to enjoy drinking. However they are very high in sugar so it is important to ensure that teeth are brushed thoroughly after consuming these (if cleaning teeth is a problem encourage with savoury nutritional supplements instead) 17
2.4
Pureed diets
Pureed diets will have a lower nutrient and energy content because of processing than a normal or soft diet. Therefore they are discussed in detail in this section and some ideas for how to enrich pureed meals are also given. As the HD disease progresses it will be necessary to alter the consistency of food for easier chewing and safe swallowing and in due course a pureed diet will be indicated. This means that additional liquid will need to be added to most food in order to obtain the required consistency. When adding a liquid use one which contains nutrients such as stock, milk, butter or gravy to obtain a good consistency. Food processors, hand blenders, sieve or a juicer are all very useful aids. Note that when more stock/fluid is added to food the volume will increase while the amount of calories per volume decreases. Nutritional content by volume may be reduced by as much as 50%. Therefore pureed diets will need to be fortified with additional calories to meet most individual’s nutritional requirements and to prevent weight loss. Following the balanced diet guide and the ideas given below for fortifying the diet will provide additional calories and nutrients simply. The estimated energy content of a pureed menu and some ideas of how to enrich it simply, into a high calorie meal are given below. Example of a puree menu consistency (C/B) Breakfast Instant oat cereal with milk tea and pureed soft fruit Orange juice Mid Morning Smooth yogurt Tea with milk and 2 sugar Lunch Puree shepherds pie, Carrots Puree pears and cream Fruit juice Mid Afternoon Tea with milk and 2 sugars Dinner
Puree lamb, potatoes, and broccoli Dessert (mousse) Fruit juice
Supper
Hot milky drink 200mls
Ideas for fortifying (adding extra calories) Use fortified milk in cereal and drinks Add sugar to taste Add cream or full fat yoghurt** to fruit Replace juice with fruit smoothie Use thick and creamy yogurt Replace tea with milk shake** Add extra butter and cream to potatoes and butter to vegetables Replace pears puree with banana /soft fruit split** (see recipe table 3) Replace tea with milk shake ** or savoury nutritional supplement Add liquidised soup with added cream Add extra butter and cream to potatoes and butter to vegetables Use ice cream** to make a Chocolate gateau smoothie** for dessert (table 3) Milk shake** and creamy dessert** or a sandwich with soft filling e.g. creamed cheese soaked in a soaking solution. (Commercial thickeners can be used as soaking solutions. They can be poured over different foods such as cakes, biscuits and sandwiches. These solutions alter the texture of food making it easier to swallow)
Approximate Energy
= 1465 kcals
Approximately Energy = 3600 kcals
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2.4.1 Further ideas for enriching a pureed diet (to advised consistency):
Add evaporated milk, double cream, condensed milk** to pureed fruits or mousse like desserts** as snacks between meals Choose extra creamy yogurts, creamy desserts** and mousses** or milk shakes/smoothie** as snacks between meals Choose rich desserts with chocolate**, cream, cream cheese Add cream cheese to mashed potatoes or soups Use fortified milk (see add extra calories) in drinks, pureed desserts, custards etc If diet alone cannot meet the nutritional needs of the patient there is a wide variety of nutritional supplements available enriched with calories, vitamins and minerals. Consult your Dietitian for the most suitable nutritional supplement and refer to the nutritional supplement flow chart in Appendix 3 for guidance. If nutritional supplements need to be thickened then refer to Appendix 2 Table 3
Food Presentation Our senses play important roles in enhancing our nutrition improving our appetite and our enjoyment of our food. For example, visual contrast may improve food and fluid intake in some individuals, so use colour full table wear and utensils if possible. Our sense of sight and smell also prompt the body to produce digestive enzymes (that are necessary for getting nutrients from food) and stimulate the appetite. Therefore the effect of the presentation of food and the aroma should not be underestimated. Keep food as visually appealing as possible as well as flavoursome and avoid serving food with no contrast of colour e.g. white fish with white potatoes on a white plate. Extra care is needed when presenting foods which have been modified, use forks, scoops, piping bags etc to present these attractively and avoid altering the flavour (16). *For some delicious recipes including texture modified diets see resources list ‘Flour of Scotland’ *Note on texture modified diets National Descriptors for Texture Modified diet in adults have been agreed jointly by Dietitians and SLT’s in the UK. Carers may find these tables describing food and fluids useful to refer to in conjunction with a Dietitian or SLT who can advise how these tables can be applied for individual clients. See Appendix 2
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Iron intake As well as calories it’s important to ensure that the full range of nutrients is provided. Sources of iron can tend to be limited in a modified texture diet so it’s important to include good sources of iron such as red meat, liver, fish, eggs, lentils, beans, soy, leafy greens like spinach, broccoli, and dried fruit. Grains such as wheat and oats e.g. oatmeal, whole wheat bread, and soy products such a tofu, tempeh and soymilk are all good sources of iron. The following list may be helpful to you when choosing meals .
Food Instant oat cereal (fortified) (30 g) Meat stew (average portion) Chick peas/Hummus 200g 2 slices of lambs liver Spinach 100g Cottage pie/20lasagne (average portion size) Small tin of beans (225g) Black treacle (35g) 2 weetabix 2 pork sausages Thick slice of corned beef 1 sardines in tomato sauce (average portion size)
Iron mg 3.6mg 6.2 6.0 4.0 4.0 3.6 3.2 3.2 3.0 1.8 1.5 1.2
An average daily required intake of iron is 8.7mg men – 14.8mg woman.
Eating out
Plan ahead. If unsure what will be available, take food with you if possible. Restaurants may be helpful if pre-warned, however the carer still needs to check for hard crusts, chunky vegetables in dishes etc. As far as possible adapt what’s available by chopping, moistening with extra sauce, ketchup, mayonnaise etc. Be discreet in making any special arrangements, such as thickening drinks, cutting up food. This may be rejected by the individual who will want to be treated like anyone else and may not acknowledge their difficulties.
2.4.3 Regular Aspects Regular meals Mealtime’s play a huge role in the daily life of people with HD. Routines are comforting and reassuring so it is important to devise a regular meal schedule and to stick to it. Regular bowels Constipation can cause loss of appetite and even vomiting. Try to include lots of fresh
fruits and vegetables and plenty of fluids (1.5l/day minimum) to keep the digestive system regular. Monitor hydration status with simple checks such as colour of urine and skin elasticity. Try to encourage gentle activity. Consider using laxatives if needed.
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Regular weight checks Weight loss is not universal but when weight loss occurs it can be very rapid. Therefore it is important to check weight regularly in order to treat weight loss promptly. If weighing is difficult and special scales are not available then using other signs such as clothing and jewellery can show changing weight. Find somewhere where hoist, wheelchair, or sitting scales may be available. Keep a record of the last accurate height measure as this will be important to calculate the body mass index, which is needed for assessments. Regular dental care High calorie snacks and drinks between meals increase the risk of dental caries (17). Plaque build up can cause dental caries and poor oral hygiene can increase the risk of aspiration pneumonia (especially for those who are fed only via long term feeding tubes). Encourage good oral hygiene for healthy teeth and gums by regular teeth cleaning (after each meal) with a fluoride tooth paste (.17). Encourage the use of a high fluoride mouthwash especially after **high sugar snacks. Apply lip salve if lips are dry. Keep dentures clean and make sure they fit properly. Regular dental check ups are important. Appropriate dental care equipment is important e.g. curved toothbrush, enlarged handles for easy grip. See EURO-Hd Dental SoC (17)) for more information on dental care.
2.5. HD and other clinical conditions affected by diet With other conditions which are affected by food some individualised advice is needed and should be sought from a Dietitian. For example for some people with HD including high calorie, high fat foods may not be necessary to keep weight stable. Brief overviews on diet and heart disease/diabetes are given below. Cardiovascular disease and raised cholesterol. Choosing unsaturated fats and soluble fibre will be helpful for heart health. There are two types of unsaturated fat that should be included in the diet for a healthy heart, monounsaturated and polyunsaturated. Monounsaturated fats Olive oil, rapeseed oil, groundnut oil and margarines made from these, and avocados and nuts. Can be used in cooking, spreading and baking. e.g. margarine on bread and in baking. Use oils in dressings, with pasta, or on roast vegetables.
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Polyunsaturated fats Sunflower, corn and soya oil, and margarines labelled as high in polyunsaturated fat. Omega 3 polyunsaturated fats are found in oily fish such as mackerel, salmon, pilchards. Try to eat oily fish every week; care is needed to remove any bones. Tinned mackerel and pilchards can be mixed with soft cheese or tomato sauce to make a sandwich filling or a topping for toast or potatoes. Fresh tuna, trout, fresh or tinned salmon can be served hot with a sauce to moisten or in a quiche, omelette, pasta dishes. Soluble fibre Peas, beans, lentils, oats and fruit are recommended. Red lentils are a good addition to stews and casseroles and cook to a soft smooth texture. Remove any tough shells on pulse vegetables by sieving e.g. mushy peas, humous. These foods are also filling and low in calories if trying to prevent weight gain (see appendix for further advice on weight management). Diabetes Keeping blood glucose levels within a safe range requires balancing food and medication. It’s important though to balance what are the most important issues at any time. Carers should work very closely with the Dietitian and the healthcare team. Medications can be adjusted according to what is being eaten at any stage. As far as possible try to continue with these principles;
Aim for regular meals which include starchy foods such as bread, rice, potatoes, pasta, especially wholegrain and high-fibre varieties.
If sweet foods are preferred and savoury foods are poorly taken, no added sugar milk based desserts such as rice pudding or mousses can be a nourishing alternative. When nutritional supplements are needed, medications can be adjusted to balance the sugar content of these.
Confectionary and sugary foods should usually be limited to small servings at any time and preferably after meals.
‘Diabetic’ foods offer no special benefit. They are usually more expensive and can also have a laxative effect.
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2.6
Advanced Decisions (AD) and Enteral Tube Feeding (ETF)
Advanced decisions are generally statements made about health care when a patient has the capacity to decide such matters. These statements can then be referred to if mental incapacity supervenes. HD in its advanced stage presents a range of issues that can make the planning and delivery of care complex and an important concern is the need to make Advanced Decisions around end of life care and in particular, Enteral Tube Feeding (ETF). Commonly the wishes of the person with HD for their end of life care, in particular ETF are not recorded in their medical notes. This can leave family members or their clinicians with having to make this decision on their behalf. It can also lead to feeding tubes being placed in a crisis situation e.g. when a patient is admitted to hospital with a chest infection or as a management option without any knowledge of the individuals wishes. Therefore it is highly recommended that AD for ETF is discussed with people with HD early in the disease process so that their wishes for long term feeding options can be documented. This dialogue should be led by the person who is closest to the individual. This may be a family member, carer or a health professional.
2.6.1 Enteral Tube Feeding (ETF) Enteral feeding literally means using the gastrointestinal tract for the delivery of nutrients, which includes eating food, consuming oral supplements and all types of tube feeding. The routes of ETF most often used are naso-gastric tubes (NGT) and percutaneous endoscopic gastrostomy (PEG) tubes. Other routes that are increasingly being used include jejunostomy feeding, which may be the only feasible route if it is not appropriate to feed via the stomach. The type of tube will depend on the intended duration of the feeding and the part of the gastrointestinal tract the feed needs to be delivered to. The following section provides some information on Enteral tubes, mode of feeding, care of tubes and describes some gastrointestinal problems and possible causes and solutions.
Types of Tube The three main types of feeding tubes are described below. Percutaneous Endoscopic Gastrostomy (PEG) is the most commonly placed feeding tube in people with HD therefore care of this type of tube is discussed in this section 1
2
3
Naso-gastric tube (NGT) is passed through a nostril down the throat and into the stomach. It is usually for temporary feeding, up to 4 weeks, and can be easily dislodged so ensuring correct position before feeding each time is essential. Due to its position it may interfere with swallowing. Percutaneous Endoscopic Gastrostomy (PEG) is inserted through the abdomen into the stomach. A permanent tract forms within 3 weeks and the device is held in place with an internal balloon or bumper and an external fixator plate (18). It is usually for longterm use and it is the type of feeding tube commonly placed in people with HD. Jejunostomy tube (J-tube) is inserted below the stomach, directly into the small intestine. Because it is placed below the stomach it reduces the risk that feed will back up into the oesophagus into the trachea and lungs (aspiration). However it does not eliminate the problem (19). Slow rate of feeding is required as it may increase the risk of diarrhoea 23
2.6.2 Mode of feed Feedings can be either "continuous" or as several "bolus" feeds. Feeding plans can be adapted according to individual tolerance and preferred daily routine: Continuous As the name implies, continuous feedings are given at a fixed rate using a pump throughout the day and/or night. Tolerance problems are usually minimized when feeds are given in this way as the rate can be adjusted and given slowly as needed. Patients at risk for regurgitation may benefit from continuous feeding.
Bolus A "bolus" is a set amount of feed given at specific times by syringe, usually at mealtimes and snack times. Large volumes may increase the risk of oesophageal reflux or diarrhoea. It may be more practical for those who are mobile or those at risk of dislodging the tube. (A bolus should be administered only into the stomach so is not used with J-tube) To minimise risks of aspiration, patients should be fed propped up at 30° or more during feed and administration of medications (20) and should be kept propped up at least 30 minutes after feeding (19).
2.6.3 Care of Percutaneous Endoscopic Gastrostomy (PEG) Tube Care Do’s and Don’ts:
Do always wash your hands and dry thoroughly before touching the feed and handling the tube. Nursing staff will use gloves when handling tube. Do wash the tube site daily with soap and water and dry thoroughly or follow any special instructions you may be given to keep the tube and the area around it clean. The site is prone to infection so check closely before every feed. Do identify the position of the tube. The tube has a marker on it to identify its position. The position of the tube should always be checked daily before each feed to ensure it has not altered. Do report any signs of infection (21). Daily checks include observing the stoma site for signs of leakage, redness, swelling and irritation, skin breakdown, soreness or excessive movement of the tube (more than 6mm) in or out of the patient’s stomach .(36). Do rotate the tube 360 degrees once a day. Do advance it in and out once a day. This is only a very small movement .(18) Do not use the tube if it has moved Contact your local healthcare professional. Do not use creams or powders unless advised by your healthcare team Do not apply a dressing around the tube unless advised by medical/nursing staff. (18) Dressings are not necessary as they precipitate moisture and irritate the surrounding skin and tissue.
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2.6.4 Percutaneous Endoscopic Gastrostomy (PEG) In the community PEG tubes are the most common and easiest to manage. Tubes are placed usually under local anaesthetic, with a small incision made in the abdominal wall, and the tube is inserted with the help of an endoscope. PEG tubes vary in width from 9-28FG, and normally last for 18 months to 2 years. When PEG tubes are no longer required, they may be removed by traction, repeat endoscopy or they can be cut and allowed to pass naturally, although this remains controversial (Rollins 1998).
Balloon Gastrostomy These are generally
only sited when a stoma has already been formed by an existing PEG insertion. There is an inflatable balloon at one end (inside the body) and an external base (outside the body) at the other. The external base holds the tube in place yet allows air to circulate around the skin. The bottom of the base should rest just above the skin surface. The balloon needs checking and re-inflating every seven days; although some more modern tubes may be checked less frequently. Balloon gastrostomy tubes generally need replacing every 2-3 months and arrangements should be made to always have a spare one available. If removed prompt replacement is crucial to avoid closure of the stoma. Always follow the manufacturers advice for the type of tube you are using.
2.6.5 Flushing the Enteral Feeding Tube (EFT) The only things that should go down an EFT are medications, feed and water (19). Always consult your local pharmacist for advice of drug administration and EFT . A patient guide to giving medications through EFT is also available to download from the internet http://www.bapen.org.uk/pdfs/d_and_e/de_pat_guide.pdf Volume Tubes should be flushed with water before and after each ‘bolus’ of feed, every 4-6 hours during continuous feed and before and after medications using a 50ml enteral feeding syringe (22). Unless instructed otherwise by your Dietitian a 15-30ml water flush is recommended before and after feed and medications. Water type will depend on local policy (23). Any medications given through a tube should ideally be in liquid form. Syrupy medications may require additional water flushes (19). Technique Prepare a flush of water (according to local policy) in a 50 ml enteral syringe Stop or suspend enteral feeding Ensure other ports are closed tight Attach the syringe to a port of the patients EFT and ensure there is an airtight connection between syringe and the EFT. Administer the flush () Administer the drug and flush; cap off, or connect further enteral feeding depending on the patients requirements (19) If the tube blocks: Deal with it as quickly as possible
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Massage any obvious blockage to break it up. Use a 50ml syringe, and gently flushing back and forward with warm water or (19) soda water may be helpful. If this fails, contact your healthcare team for further advice. Do not use colas, pineapple juice or other solutions with acidic pH Alkaline solution of pancreatic enzymes (24) or ‘Clog Zapper’ a patented enzyme powder may be useful but these are expensive and only useful if feed has blocked the tube.
2.6.7 Use of clamps
Some PEG tubes have a clamp on the tube to prevent leakage when the cap at the end of the tube is removed. This clamp should be left in the open position – except when the cap is being opened or closed in between feeds, flushing or delivery of medications. If the clamp is in the closed position for long periods of time this will damage the tube (18).
2.7. Enteral Tube Feeding (ETF) Gastrointestinal Problems in HD The most common complications of tube feeding and HD individuals are of a gastrointestinal nature including vomiting, diarrhoea and constipation (28). This section highlights some possible solutions that you can discuss with your Dietitian and other health care providers.
Please note however if the person shows signs of being unwell, it is important to check with the healthcare team for any other causes. People can quickly become dehydrated with vomiting and diarrhoea and fluids will need to be replaced,
2.7.1. Vomiting/Gastrointestinal reflux (GORD) Check: Is the rate of feed too fast? Is the bolus too big? Is the feed too cold? Is the person receiving the feed lying too flat while feed is running? Has the type of feed recently changed? Is the patient constipated? Try:
Reduce the flow rate (in consultation with your Dietitian) Reduce the bolus size (in consultation with your Dietitian) Give feed at room temperature Ensure patient is in correct position for feeding and not lying flat for at least an hour after the feed Treat constipation if present *Medication for reflux may be useful e.g. omeprazole *Medications to increase the motility of the stomach may be useful e.g. domperidone 26
2.7.2 Diarrhoea Check: Is the rate of feed too fast? If normally on regular laxatives check that these have been stopped. Diarrhoea may be caused by taking antibiotics. Do not stop the antibiotics if they are prescribed. If the diarrhoea continues contact your GP local health care provider and Dietitian for further advice. Check that all infection control measures are being followed according to local policy. (21)
Try:
If the rate has recently been increased. Reduce back to last tolerated rate and contact your Dietitian Fibre containing feeds sometimes help ETF related diarrhoea, as will breaks in continuous feeding of 4–8 hours. (18). Discuss with your Dietitian if patient is not on a fibre containing feed or if the feed is being administered without a break of 4 hours or more. Whenever diarrhoea occurs with ETF, consult with your GP or pharmacist for a review of medications especially laxatives and drugs containing magnesium such as antacid preparations or sorbitol. Sorbitol ≤15glday will have a laxative effect. (19 )
2.7.3 Constipation Check:
Does the feed contain fibre? Have the full amount of fluids been given? (Refer to your feeding instructions). Has there been any recent loss of fluids due to excessive sweating (people with HD can often have very high body temperatures and increased requirements for fluids) or fluid losses through vomiting or recent diarrhoea? If so contact your Dietitian for advice.
Try:
Ensure the patient is getting the amount of fluid advised by the Dietitian Regular laxatives may be required if mobility is reduced
Bloated during feeding:
Check the feed is not being given too quickly. Open the feeding tube cover to release any trapped wind.
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Part 3:
Notes for Practicing Dietitians
Observation has shown that there can be variations in energy requirements throughout the course of the disease and between HD individuals (25) but generally speaking, in the early stages of HD patients may have higher energy requirements than the normal population ( 26, 27) and this may explain why they are often underweight. However there is greater variability in energy requirements as the disease progresses (28) and in the later stages of HD energy requirements appear to become lower (29) which may be due to reduced mobility. Due to the variability in energy requirements and the potential for rapid weight loss, early individual assessment and regular reviewing of nutritional care plans are vital. . In a multidisciplinary setting it may not be necessary to request all of this information if it has previously been documented by others and if the information is readily available to the Dietitian.
3.1
HD Dietetic Assessment Framework
A background history allows the Dietitian to:
Gain subjective information on the patients presenting problems Place this information in the context of their past medical history and current condition Document how their presenting problems may affect their ability to live independently Address the patient holistically (13)
It is recommended these aspects should be documented in a comprehensive nutritional assessment.
Patient information and demographics. Information as to the date of diagnosis and medical history as well as family history should be obtained. A list of any medications taken for HD (medications may affect saliva, appetite, gastrointestinal problems (see Appendix 5 for guidance) as well as for any other medical condition. The reason for referral as well as others who are involved in their care e.g. social worker, clinicians, therapists Current weight and weight history Level of activity and estimated energy requirements Individual’s food preferences Ability to chew and swallow food and fluid Ability to feed themselves/food spillage Problems with constipation, reflux Assess the need for referral to other professionals e.g. SLT, Physiotherapist, OT, Dentist Daily routine and sleeping pattern Shopping and cooking arrangements, and ability to plan Behavioural health risks e.g. alcohol intake, drug use/smoking Attitude to food and body weight
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Social history
Social situation, level of support e.g. home environment; family support Current or previous employment Hobbies, interests, social activities, religious belief impacting on diet Family history of HD General health perception/quality of life Future wishes, fears
It is essential to have the views of both patient and carers when formulating nutritional plans. The assessment should be reviewed on a regular basis, as the various stages of the disease present different nutritional challenges (1).
3.2 Nutritional care plan Developing and documenting a Nutritional Care Plan for an individual with HD should include the following: Goal Setting The following are general guidelines for setting goals for people with HD:
Goals should address specific problems that are amenable to Dietetic intervention Seek understanding and agreement from the patient Goals should be measurable
Intervention Dietary recommendations will take into account the issues described in detail in previous sections as well as (EURO-Hd), Standards of Care (SOC) Nutritional Guidelines for People with HD. Coordination & Communication Describe all coordination and communication that needs to take place with other professionals, caregivers, or family members so that nutritional care plans can be implemented Patient related instruction This should include any advice given to the patients or their caregiver and any planned patients/carer education. It will also include any planned changed to diet, the use of nutritional supplements etc. Review A care plan review date should be agreed with the patient/carer and documented
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Note In the early stages the individual may be just be coming to terms with their diagnosis, and might not be willing or ready to accept advice or recommendations. They may also have difficulty discussing what the future holds. It is important for the therapist to be sensitive to the individual’s emotional state. It may necessitate progressing more slowly with an intervention plan than might ideally be indicated (13)
3.3
Multidisciplinary working
Whilst Therapists cannot delay the progress of the disease, there is much that can be achieved by working together to assist the patients with every day problems and needs. Therefore the importance of multidisciplinary working cannot be overemphasised (30). Speech and language therapists have skills in assessing and making recommendations for a safer swallow and maximizing the individual’s ability to communicate. They can advise on the most appropriate textures of food and fluids and the use of communications aids. Occupational Therapists (OTs) can review the body position at meal times; suggest adaptations to seating or changing the cutlery or advise on other aides to eating. Physiotherapist involvement can include working closely with OTs and looking at the individual’s ability to maintain posture while seated at mealtimes. They may work on exercises to improve the patient’s trunk stability or body position while seated. They can also advise on how to maintain chest status at times when chest infections are problematic. The integrated approach of therapists working together provides each therapist with a broader understanding of the individual’s needs, and results in the ability to provide a more effective degree of treatment and care. Other interdisciplinary professionals include geneticists, neurologists, nurses, psychologists and social workers among others. It is critical for therapists to consider collaboration with these professionals to understand their role as part of an interdisciplinary team (13)
3.4.
Making an informed choice on Enteral Tube Feeding (ETF)
It must always be remembered that it is the individual’s decision as to whether tube feeding is appropriate for them or not. They and their family should never be persuaded to make a choice but rather they should be given enough information to make an informed choice. Their decision must be respected and supported by all health professionals involved. Information should be given before any cognitive changes and in a manner that is appropriate to their understanding. This will allow people with HD to reach a decision based on their own values (31). The Dietitian is ideally placed to provide this information in a timely manner (though it may be provided by another health professional e.g. named nurse). Help the individual make an informed choice by discussing the following points:
P.E.G. What it is? Petutaneous Endoscopic Gastrostomy (PEG) is commonly called a PEG. It is a way of providing liquid food directly into the stomach (Show examples of PEG tubes that the patients can handle and examine or use simple diagrams or video).
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How is it placed? An assessment is carried out first to ensure that you are medically fit for the procedure. The procedure itself involves a tube called an endoscope which is passed into your stomach. A small cut is made in the abdomen and the PEG is placed through the abdominal wall into the stomach
Where is it placed? It is a minor operation and is usually done under a local anaesthetic or a light sedative at your local hospital. You will feel sleepy and unaware of what is happening. The procedure takes less than an hour and you may have to stay in hospital for a few days to make sure the PEG is working and to allow the Dietitian to organise your feeding plan
How does it feed me? A liquid feed which contains all the nutrients that you require is used. Feedings can be either "continuous" or "bolus" servings. Continuous feedings run at a fixed rate throughout the day and/or night using a pump. A "bolus" is a set amount of feed given down the tube at specific times during the day e.g. mealtimes and snack times. It may be more practical for those who are mobile or those at risk of dislodging the tube. Some people are fed totally via the PEG while others can continue to take some food or fluids by mouth after the tube is fitted.
How will it be cared for? A PEG tube needs daily care, for cleaning and to check it stays healthy. You will be offered support from a carer or health care professional.
Why consider it? Difficulties with eating and drinking and weight loss are common in HD. As the disease progresses these difficulties may become harder to manage and there is an increased risk of food going down the wrong way (aspiration) and choking. Food or fluids entering the lungs, can lead to chest infections. If this occurs often you may want to consider a different way to get nourishment. Having a PEG does not entirely remove the possibility of aspirating and some people choose not to have a PEG and continue to eat and drink despite the risk
What problems might I have? Any problems with PEG or the PEG site can be managed with help from your Dietitian and healthcare team. The PEG may leak causing pain or infection around the PEG site It might become blocked It might be accidentally pulled out 31
What are the benefits? It may enable you to spend less time and effort eating and drinking and you may be able to engage more in other activities that you enjoy With having less pressure to meet all your needs by eating and drinking, mealtimes can become more relaxed and focused on eating for pleasure It will reduce the incidence of coughing/choking at mealtimes It will maximise your nutritional intake and reduce the risk of dehydration
Important points to communicate: It does not delay the progression of HD It may prolong life?
3.5
Enteral Tube Feeding (ETF) Decision Making Check List
In some cases the placement of a feeding tube may be not be in the best interests of the individual. Therefore, the "right" decision requires that everyone involved makes every effort to make their contribution to this decision be as informed as possible. If there is no AD regarding ETF and the patient does not have the capacity to make a decision, then placement of a feeding tube should only be considered if the following areas have been fully explored
Adequate training has been given to all those involved in feeding Review of positioning /seating while feeding has been explored Review of the consistency of the food and fluids A wide variety of nutritional supplements have been tried Other reason for swallow deterioration that can be alleviated with therapy have been ruled out ( anxiety or an infection e.g. urinary tract infection ,both of which can affect swallowing temporarily) Medications have been considered and reviewed (some can cause dry mouth and affect swallow). A greater degree of relaxation while eating has been explored e.g. the interaction between the person and those helping him to eat, the eating environment, passive limb exercises before meals. Consideration of any other psychological factors that may be alleviated with therapy.
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Appendix 1 Avoiding Excessive Weight Gain For some people with HD unintentional weight gain leading to being overweight can be a problem, often linked to reduced mobility and /or eating more. Excessive weight gain can cause difficulties, for example with using some equipment and moving around as well as affecting self esteem so should be avoided. The following offers some basic healthy eating advice but ideally individual advice should be sought from a Dietitian or other health professional with knowledge of HD.
To avoid further weight gain, or if necessary to lose weight, both diet and activity should be considered with the aim to make small but long term changes. Reduce calories from high sugar and fat sources , though an occasional treat is acceptable. Other nutrients such as vitamins and minerals are still very important for good health, so eating balanced, regular meals is essential and meals should not be missed.
To reduce sugars try the following: Offer fewer sugary drinks and snacks. Instead of sugary carbonated drinks and sweetened juice drinks, go for water, low calorie diluting or carbonated drinks. Fruit juice can also be diluted with sparkling water. Instead of cakes or biscuits, try having fruit, fresh or cooked, a yogurt, teacake or currant bun. If sugary hot drinks are preferred try adding artificial sweeteners instead of sugar. Artificial sweetener can also be added to breakfast cereals. Rather than offering jam, marmalade, syrup, treacle or honey on bread, try a low-fat spread, sliced banana, marmite or low-fat cream cheese instead. Offer foods with less added sugar or go for the low-sugar versions. Offer fruit in juice rather than syrup. Offer wholegrain breakfast cereals rather than those coated with sugar or honey. To reduce fat intake try the following: Grill, bake, poach or steam food, rather than frying or roasting, so no extra fat is added. Trim visible fat and skin off meat. Choose lower-fat dairy products when you can. Put more vegetables or beans in casseroles, stews and curries, and a bit less meat – and skim the fat off the top before serving, if you can. Use less oil when cooking by measuring with a tablespoon rather than pouring it straight from a container When you're making sandwiches, try leaving out the butter or spread – you might not need it if you're using a moist filling e.g. with low fat mayonnaise. When you do use spread, go for a reduced-fat variety and choose one that is soft straight from the fridge so it's easier to spread thinly.
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Appendix 2 Dysphagia Diet Food Texture Descriptions These descriptors were developed by the National Patient Safety Agency (NPSA) Dysphagia Expert Reference Group in association with Cardiff and Vale University Health Board. It included representatives from nursing, speech and language therapy, dietetics, hospital catering and industry. These new 2011 descriptors have been endorsed by the BDA, RCSLT, Hospital Caterers Association (HCA) and the National Nurses Nutrition Group (NNNG). The scientific evidence in this field is limited. These descriptors are therefore based on the best available current evidence, on texture descriptors produced internationally, and a consensus of expert opinion. They were developed to address concern related to patient safety and a request from caters for guidance. Carers may find these tables useful to refer to in conjunction with caters and Dietitian’s. SLT can advise on how these tables can be applied for individual’s clients.
Food Table 1 B. Thin puree dysphagia diet
Texture check
√ Food has been puréed or has purée texture. It does not require chewing. √ It is a thin purée (*please see note below). √ It is smooth throughout with no ‘bits’ (no lumps, fibres, bits of shell/skins, bits of husk, particles of gristle/bone etc). It may need to be sieved to achieve this. √ It may have a fine ‘textured’ quality as long as the bolus remains cohesive in the mouth. √ It is moist. √ Any fluid in or on the food is as thick as the purée itself. ×There are no loose fluids that have separated off. × The texture is not sticky in the mouth. × No garnish.
Does not hold its shape on a plate or when scooped Cannot be eaten with a fork because it slowly drops through the prongs The prongs of a fork do not make a clear pattern on the surface It can not be piped, layered or moulded ‘Spreads out’ if spilled A light, disposable plastic teaspoon is able to stand upright when the head is fully covered. If it does not do this, the texture is too thin.
Check before serving/eating: × No hard pieces crust or skin has formed during cooking/heating/standing. × It has not thinned out and any liquid within the food has not separated off. Note: No ice cream or jelly unless advised as suitable by speech & language therapist on an individual basis.
C. Thick puree dysphagia diet √Food has been puréed or has purée texture.
Holds its shape on a plate when scooped 34
It does not require chewing. √ It is a thick purée (*please see note below). √ It is smooth throughout with no ‘bits’ (no lumps, fibres, bits of shell/skin, bits of husk, particles of gristle/bone etc.) It may need to be sieved to achieve this. √ It may have a fine ‘textured’ quality as long as the bolus remains cohesive in the mouth. √ It is moist. √ Any fluid in or on the food is as thick as the purée itself. × There are no loose fluids that have separated off. × The texture is not sticky in the mouth × It is not rubbery. × No garnish. Check before serving/eating: × No hard pieces crust or skins have formed during cooking/heating/standing. × Fluid/gravy/sauce/custard in or on the food has not thinned out or separated off.
Can be eaten with a fork because it does not drop through the prongs The prongs of the fork make a clear pattern on the surface It can be piped, layered or moulded Cannot be poured. Does not spread out if spilled Example Breakfasts and Desserts The texture of thick smooth porridge made from powder (purée porridge) with no loose fluids The texture of wheat-biscuit breakfast cereal fully softened with milk fully absorbed The texture of thick blancmange or mousse with no ‘bits’ The texture of purée rice pudding There are no lumps There are no loose fluids Note: No ice cream or jelly unless advised as suitable by speech and language therapist on an individual basis.
D. Pre- Mashed Dysphagia Diet √ Food is soft, tender and moist. Needs very little chewing. √ It has been mashed up with a fork before serving (see overleaf for meat). √ It usually requires a very thick, smooth (non-pouring) sauce, gravy or custard (see next point). √ Any fluid, gravy, sauce or custard in or on the food is very thick must hold its shape on a plate and can not be poured)). × No mixed (thick-thin) textures. No loose fluid. × No hard, tough, chewy, fibrous, stringy, dry, crispy, crunchy or crumbly bits. × No pips, seeds, pith/inside skin. No skins or outer shells e.g. on peas, grapes. No husks. × No skin, bone or gristle. × No round or long-shaped foods e.g.
Example breakfasts The texture of thick smooth porridge made from powder (purée porridge) with no loose fluids The texture of wheat-biscuit breakfast cereal fully softened with milk fully absorbed The texture of thick blancmange or mousse with no ‘bits’ The texture of purée rice pudding There are no loose fluids There are no lumps Wheat-biscuit breakfast cereal has not fully softened Milk has not fully absorbed leaving loose fluid Example Desserts 35
sausages, grapes, sweets. No hard chunks e.g. pieces of apple. × No sticky foods e.g. cheese chunks, marshmallows. × No ‘floppy’ foods e.g. lettuce, cucumber, uncooked baby spinach leaves. × No juicy food where juice separates off in the mouth to a mixed texture e.g. water melon. Check before serving/eating: × No hard pieces crust or skins have formed during cooking/heating/standing. × Fluid/gravy sauce/custard in or on the food has not thinned out or separated off. *Note – definition of ‘very thick’ fluid Any fluid, gravy, sauce or custard in or on food must be very thick. It holds its shape on a plate or when scooped, can not be poured and does not ‘spread out’ if spilled. Thinner single texture foods maybe suitable if a person is on thinner fluids –SLT to advise on individual basis
The texture of very thick, smooth yogurt (no bits) or stewed apple in very thick custard. Or the texture of soft sponge cake with smooth filling fully softened by mashing and mixing in with very thick, smooths (non-pouring) custard. Overall texture must be very thick. Additional foods Meat finely minced, fish mashed smooth and both should be served in thick smooth sauce. Casserole/stew must be very thick and tender. Fruit mashed and drained. No bread unless assessed as suitable by SLT on an individual basis Note: No ice cream or jelly unless advised as suitable by speech and language therapist on an individual basis.
E. Fork Mashable Dysphagia Diet
Must be in a consistency that allows them to be mashed easily at point of consumption √ Food is soft, tender and moist but needs Meat some chewing. Pieces of soft tender meat must be served no √ It can be mashed with a fork. bigger than 15mms. √ It usually requires a thick, smooth sauce, Or serve meat finely minced. No hard bits of mince. Serve in a thick smooth sauce or gravy or custard (see next point). √ Any fluid, gravy, sauce or custard in or on gravy. the food is thick (*please see note below). For children × No mixed (thick-thin) textures. No thin loose Specific guidance on the size of the meat fluid. particles based on the child’s swallowing × No hard, tough, chewy, fibrous, stringy, dry, skills, their age and their development level crispy, crunchy or crumbly bits. will be provided by a speech and language × No pips, seeds, pith/inside skin. No skins or therapist following individual assessment. outer shells e.g. on peas, grapes. No husks. • Fish × No skin, bone or gristle. Soft enough to break up into small pieces with × No round or long-shaped foods e.g. a fork. Serve in thick smooth sauce or gravy. sausages, grapes, sweets. No hard chunks e.g. pieces of apple. • Fruit × No sticky foods e.g. cheese chunks, Juicy fruit should be mashed – drain away any marshmallows. juice that has separated. × No ‘floppy’ foods e.g. lettuce, cucumber, • Casserole/stew/curry Must be thick. Can contain meat, fish or uncooked baby spinach leaves. × No juicy food where juice separates off in vegetables if prepared as above and overleaf the mouth to a mixed texture e.g. water and fully mixed in. 36
melon. Check before serving/eating: × No hard pieces crust or skins have formed during cooking/heating/standing. × Fluid/gravy/sauce/custard in or on the food has not thinned out or separated off. *Note – definition of ‘thick’ fluid Any fluid, gravy, sauce or custard in or on food must be thick – a light disposable plastic teaspoon would stand upright if the head were fully but just covered. Those on Texture E must therefore be able to cope with thinner fluids – stage 2 (see table 2)
For those with the HD gene who are not on a texture modified diet some food can cause coughing and increase the risk of choking and therefore should be avoided. See Foods to avoid
• Bread No bread unless assessed as suitable by SLT on an individual basis. • Cereal The texture of thick smooth porridge with no lumps. Or the texture of fully softened wheat-biscuit breakfast cereal with milk fully absorbed. Any milk/fluid must not separate off (i.e. no thin loose fluid/no mixed (thick- thin) textures. Overall texture must be thick (Because this is a single texture food it could be served thinner if a person is on thinner fluids – SLT to advise). • Desserts The texture of thick smooth yogurt (no bits) or stewed apple in thick custard. Or texture of soft sponge cake with smooth filling, fully softened with thick smooth custard. Overall texture must be thick. (If the texture of the dessert is single it could be served thinner if the person is on thinner fluids – SLT to advise). No ice-cream or jelly if a person requires thickened fluids (because these can change to normal fluid thickness in the mouth). Foods to avoid Stringy, fibrous texture, e.g. pineapple, runner beans, celery, lettuce. Vegetable and fruit skins including beans, e.g. broad, baked, soya, black‐eye, peas, grapes Mixed consistency foods e.g. cereals which do not blend with milk, e.g. muesli, mince with thin gravy, soup with lumps. Crumbly items, e.g. bread crusts, pie crusts, crumble, dry biscuits. Hard foods, e.g. boiled and chewy sweets and toffees, nuts and seeds. Husks, e.g. sweet corn and granary bread. Seeds.
Adapted from Dysphagia Diet Food Texture Descriptors April 2011
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TEXTURE MODIFICATION – FLUID Table 2 DESCRIPTION OF FLUID TEXTURE
TEXTURE Thin Fluid
Still Water
Naturally Thick Fluid
Product leaves a coating on an empty glass.
Thickened Fluid = Stage 1 =
Stage 2 =
Stage 3 =
Fluid to which a commercial thickener has been added to thicken consistency. Can be drunk through a straw Can be drunk from a cup if advised or preferred Leaves a thin coat on the back of a spoon Cannot be drunk through a straw Can be drunk from a cup Leaves a thick coat on the back of a spoon Cannot be drunk through a straw Cannot be drunk from a cup Needs to be taken with a spoon
FLUID EXAMPLE Water, Tea, Coffee without milk, diluted squash, spirits, and wine. Full cream milk, Cream Liquors, Complan, Build Up (made to instructions), Ensure Plus, Fortisip, Commercial sip feeds Notes. Thickener should be added to all fluids e.g. soups alcohol, and liquid medications. Let hot drinks cool slightly before adding thickener. Thickened fluids stay in the mouth a little longer than normal fluids so avoid over flavoured drinks (add a little extra water to cordials). Always follow manufacturers/ SLT/Dietitans advice. Keep the thickener as dry as possible by replacing the lid promptly and always using dry scoops or spoons.
British Dietetic Association: National Descriptors for Texture Modification in Adults, April 2009
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A Guide to Thickening Supplement Drinks
I Many patients who require modified fluids may also require nutritional supplements with thickener added. When thickening supplement or fluids with high fat e.g. milk try the following:
Put the required amount of thickener into a shaker. Add the supplement drink to the shaker, put the lid on and shake.
Alternatively, add a small amount of the supplement drink to the thickening powder to make a paste, and then gradually mix the rest of the drink with the paste. Allow to stand for a minute (do not add more thickener) Ideally consume within 10-15 minutes as they may continue to thicken over time. Check drink is of the correct consistency before serving (Table 2). * This is a guide only always refer to manufacturers instructions when making thickened drinks
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Appendix 3 Soaking Solution Commercial thickeners can be used as soaking solutions. They can be poured over different foods such as cakes, biscuits and sandwiches. These solutions alter the texture of food making it easier to swallow Method: 1. Spread sliced white or brown bread with a suitable smooth filling. 2. Roll the bread as if making a Swiss roll. 3. Anchor carefully all the way along the roll with cocktail sticks at regular intervals about 1cm apart. 4. Cut in between each cocktail stick and soak thoroughly with the soaking solution. Chill for 2 hours, check thorough soaking has taken place and then serve.
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Appendix 4
A Practical Guide to Choosing Nutritional Supplements for People with Huntington’s Disease
Appendix 4
Adapted from PCDG 2009 (15)
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Appendix 5 Medications Medications may be prescribed for certain symptoms of HD such as chorea, depression, aggression, and temper outbursts. Unfortunately, side effects that may impact on nutritional status can accompany medications such as, dry mouth, gastrointestinal problems, constipation, weight changes, and sedation. Over-medicating is a risk as well and medications should be reviewed regularly. Persons with HD often become unable to tolerate as much alcohol (or other sedative medications) as they could in the past. This is especially important for persons who are still driving. Class of Drug (main action) (Bonelli et
Subclass of drug
Example medication
Potential side effects
Neuroleptics and atypical antipsychotics
Tiapride (Tiapridex, Synthelabo) Fluphenazine (Prolixin) Risperidone Risperdal) Olanzapine (Zuprexa) Pimozide (Orap) Geodon (Ziprasidone) Supiride Clonazepam (Klonopin); Diazepam (Valium); Tamazepam (Restoril) Tetrabenazine (Nitoman)
Drowsiness, apathy, extra-pyramidal symptoms, dystonia, akathisia (restlessness), hypotension, dizziness, headache, insomnia, constipation, dry mouth, weight gain, tardive dyskinesia
al.2004;HDSA 2000 33,3,354)
Antichoreic
Benzodiazepines
Dopamine Depleting Agent
Antipsychotic
Olanzapine (Zyprexa)
Haloperidol (Haldol)
Sedation, ataxia, apathy, withdrawal, seizures, fatigue
Hypotension, drowsiness, depression, gastrointestinal disturbance, extra-pytamidal symptoms Drowsiness, parkinsonism, apathy, akathisia (restlessness), raised tryglycerides, weight gain from increased appetite, Caution should be used with patients with diabetes and blood glucose monitored hypotension Drowsiness, apathy, extra-pyramidol 42
Class of Drug (main action) (Bonelli et
Subclass of drug
Example medication
Potential side effects
al.2004;HDSA 2000 33,3,354)
Antidepressant
Selective serotonin reuptake inhibitors(SSRI) Tricyclics
Other
Antiepileptics
symptoms, dystonia, akathisia (restlessness), hypotension, constipation, dry mouth, weight gain, tardive dyskinesia Rispiridone As Oolanzapine but less (Risperdal) affect on increasing appetite Fluphenazine Sedation, extra-pyramidal (Prolixin) symptoms, dystonia, akathisia, hypotension constipation, dry mouth, weight gain Fluoxetine (Prozac) Insomnia, diarrhoea, Sertraline (Zoloft) gastro-intestinal upset, Paroxetine (Paxil) restlessness, weight loss, Citalopram (Celexa) dry mouth, anxiety, headache Nortriptyline Insomnia, diarrhoea, (Pamelor), gastro-intestinal upset, Amitryptiline (Elavil) restlessness, weight loss, dry mouth, anxiety, headache Nefazodone Sedation, nausea,, dry mouth, dizziness, constipation Buproprion Seizures, agitation, dry mouth, insomnia, nausea Venalafaxine Hypertension, nausea, headache, constipation Valproate (Depakote) Nausea, vomiting, weight Topiramate gain or loss, cognitive Topamax) effects, Carbamazepine tremor, elevated liver (Tegretol) enzymes
For more information on medications used in the management of HD refer to: A Physicians guide to the management of HD. HAD 2009; R.A.C. Roos, et al. De ziekte van Huntington. Begeleiding en behandeling' (Huntington's disease. Counseling and treatment),. Elsevier gezondheidszorg, Maarsen 2002. (34)
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References Number 1
2 3 4
5 6 7 8 9 10
11
12 13 14 15 16 17
18 19 19 20 21 22 23 24
Title Brotherton A, Campos L, Rowell A,. Nutritional Management of individuals with HD: Nutritional Guidelines. EURO-Hd Network, Standards of Care Dietitian’s WG. 2010. Quarrell O. Huntington’s Disease-the facts. Second Edition, Oxford University Press, Oxford. 2009. Quarrell O & Cook B. Huntington’s Disease. Physical management of neurological rehabilitation. Second Edition, London, Elsevier Mosby.2004 Hamilton JM, Wolfson T, Peavy GM, Jocobson MW & Corey-Bloom J. Rates and correlates of weight change in Huntington’s disease. Journal of Journal of Neurology, Neurosurgery & Psychology, 75, pp. 209-212. 2004 Aubeeluck A & Wilson E. Huntington’s Disease. Part 1: essential background and management. British Journal of Nursing, 17(3), pp. 146-151. 2008 Folstein SE. Huntington’s Disease: A Disorder of Families. The John Hopkins University Press: Baltimore 1989 Pollard J.”Hurry up and wait! “A Cognitive Care Companion; Huntington’s Disease in the Middle and More Advance Years. 2008 HAD 2009 Lynn Rhodes. Coping with Speech and Swallowing Difficulties in Huntington’s Disease.: A Guide for Families by, MS, CCC-SLP .HDSA booklet Paulson, JS. Understanding behaviour in HD. A practical guide for individual’s families and professionals coping with HDF. Huntington’s disease society of Canada. 1999 The Swallowing Disorder in Huntington’s Disease: A Guideline Document for Speech and Language Therapists working with Huntington‘s Disease patients. . EURO-Hd Network, Standards of Care Speech and Language WG. 2010. A Guideline for Occupational Therapy in Huntington’s disease. EURO-Hd Network, Standards of Care Occupational Therapist’s WG. 2010. Quinn L, Busse M. Physiotherapy Guidance Document: For Physiotherapists. EURO-Hd Network, Standards of Care Physiotherapists WG. 2009 Huntington’s Disease Association. Eating and Swallowing Difficulties. HAD Fact sheet 6, p11 Palliative Care in Dementia Group. Sec2;p18 2009 Flour of Scotland.. A collection of inspired Scottish Recipes. Scottish Huntington’s Association A Guideline for Oral Health Care of Adults with Huntington’s disease. EURO-Hd Network, Standards of Care Dentists WG. 2010. Duncan HD, Silk DB. Problems of treatment—enteral nutrition. In: Nightingale J, ed. Intestinal failure. London: Greenwich Medical Media Ltd, 2001:477–96.) Rebecca White and Vicky Bradnam. Handbook of Drug administration via Enteral Feeding Tubes: Choice of medication formulation Chap 6; p24. Pharmaceutical Press. 2007 Rebecca White and Vicky Bradnam. Handbook of Drug administration via Enteral Feeding Tubes: Flushing Enteral feeding tubes Chap 3; p11. Pharmaceutical Press. 2007 Metheny et al. Risk factors for aspiration. Journal Parenteral Enteral Nutrition. 2002;26 (Suppl):S26-S33 NICE guidance.CG2 Infection control: information for patients, their carers and the public. June 2003 McAtear CA. Current perspectives on Enteral Nutrition in Adults, Maidenhead, Berks: British Association for Parental and Enteral Nutrition;1999 BAPEN Wyler AR, Reynolds AF. An intracranial complication of nasogastric intubation. J
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Neurosurg1977; 47:297–8. In Gut 2003;52:vii1-vii12 Wilson J. Gastrostomy feeding in advanced Huntington’s disease. Journal of Human Nutrition and Dietetics (1999), 12, 61-67. Trego A et al. Assessment of the nutritional status of patients with Huntington’s disease. Nutrition 2004.;20:192-6 Prattley R.E et al. Higher Sedentary Energy Expenditure in Patients with Huntington’s Disease. Ann Neurol 2000;47;64-70 Gaba et al. Energy balance in early-stage Huntington’s disease. American journal of Clinical Nutrition. Vol 81, No6, 1335-1341. June 2005 Nutrition and Huntington’s Disease. A Practical Guide; A Multidisciplinary Approach. Atlant Zorggroep, Apeldoornand Beekergen. The Netherlands 2000 NICE 2006. MDT working Matheson, KY and Simpson, SA Improving care at end of life: How documenting wishes can enable informed decision making Dysphagia Diet Food Texture Descriptors. National Patient Safety Agency. April 2011 Bonelli RM, Wenning GK & Kaphfammer HP (2004) Huntington’s Disease: present treatments and future therapeutic modalities. International Clinical Psychopharmacology, 2, pp.51-62 A Physicians guide to the management of HD. Huntington’s Disease Association U.K. 2002 updated 2009. Roos RAC, et al. De ziekte van Huntington. Begeleiding en behandeling',. Elsevier gezondheidszorg, Maarsen 2002. Mandy Howell, Do nurses know enough about percutaneous endoscopic gastrostomy? 23 April, 2002 Vol: 98, Issue: 17, Page: 40
Some Web resources
The following is a list of web-based and printed resources available to assist those with HD their carers in learning more about the disease process. This is by no means a comprehensive list but is a good springboard from which to find additional information. The view expressed on websites reflects the views of individuals and not the specific views of European Huntington’s Disease Network (EHDN). Fact-sheets/HD-Standards-Of-Care www.hda.org.uk/. Advice/recipies/’Flour of Scotland’ www.hdscotland.org Research in plain language www.huntington-assoc.com/hdbuzz.htm Research Network www.eurohd.net Latest news on HD http://www.hdlighthouse.org/ Information and stories http://www.hdac.org Patient care and management. http://www.kumc.edu/hospital/huntingtons PEG information http://www.hdscotland.org/images/stories/newpegbookletnov09.pdf Swallowing difficulties http://huntingtondisease.tripod.com/swallowing/id10.html Hyperlinks and other websites about HD. International hd association .
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